The whole story seems almost too cinematic to be true, but Jane Michaels swears this is how it really happened.

• Act 1: On April 1, 2007, Michaels guides her wheelchair-using daughter Delaney, then 12, through Philadelphia International Airport to begin therapy at Children's Hospital of Philadelphia for a mysterious and debilitating condition called chronic regional pain syndrome.

• Act 2: Three weeks into eight-hour daily physical therapy sessions, in which practitioners prod and cajole Delaney to work through pain that has afflicted many of her joints, Delaney falls on her face. She gets up, falls again. And again. They don't let her quit. She finally finishes at 8 p.m., 12 hours after she started.

• Act 3: Three days later, after what her doctors call a medical breakthrough in nerve retraining and what Jane Michaels calls a miracle, Delaney is discharged. She and her mother hop in a taxi and head to the Philadelphia Museum of Art. There, in homage to the famous scene from the movie "Rocky," Delaney runs up the steps, all 72 of them.

"You could almost imagine the music playing," Jane says.

• Coda: More than a year later, with her two-year bout with joint pain behind her, Delaney, now 14, is enjoying her freshman year at Folsom High School. She plays the flute in the school's marching band and participates in Irish dance competitions.

It may be too soon to say there's a definitive happy ending to Delaney's medical odyssey with a condition about which little is known or understood. There's always a chance for joint pain – feet, knees, hips, in Delaney's case – might return. And, in fact, she has had one "flare-up" in the past year.

"It was really scary because we just didn't know what it was and we didn't know whether it would go away ever or get worse," Delaney says. "It's hard to explain."

That's true even for medical professionals, who cannot even agree on a single name for the condition. It's variously called reflex sympathetic dystrophy or reflex neurovascular dystrophy or amplified musculoskeletal pain or chronic regional pain syndrome.

By whatever name, it can be debilitating for both adults and children.

According to Dr. David D. Sherry, director of clinical rheumatology at Children's Hospital of Philadelphia, no two cases are alike in manifestations. But, he adds, the syndrome is linked to a short-circuit of pain nerves in the spinal cord that sends a signal not just to the brain but also to neurovascular nerves flowing through blood vessels. That constricts blood flow and causes pain that amplifies in areas that are not injured.

Of the three major factors researchers believe trigger the syndrome – injury, illness, stress – injury is said to be the most prevalent.

"The symptoms develop immediately or within a few weeks after the injury," Sherry writes on the Web site for the Childhood RND Educational Foundation, www.childhoodrnd.org. "...The pain continues for weeks to months even though the injury is fully healed."

To this day, the Michaelses aren't sure what caused Delaney's condition.

She had, to say the least, an unlucky month in May 2006 as she was completing sixth grade: a bee sting on the bottom of her left foot that got infected; a sprained ankle playing indoor soccer; strep throat; a tetanus shot. And, in a bizarre event, she got wedged into a play structure at Fairytale Town in Sacramento and needed paramedics to free her.

Shortly thereafter, inexplicable pain began in Delaney's hips. It would come and go. Then, it traveled to her feet. Then her knees. At times, her skin became so sensitive that it hurt to the touch. Loud noises also caused her physical discomfort.

The Michaelses' pediatrician was baffled. Delaney was referred to pediatric rheumatologist and neurologists, who put her through a battery of blood tests, MRIs and bone scans.

A rheumatologist finally told Jane her daughter had "chronic regional pain" and prescribed light physical therapy at a local pool. Many days, Delaney's pain was so bad that Jane carried her from the car to therapy.

"I thought he was just using adjectives to describe the type of pain Delaney was having," Jane recalls. "The doctor never added the 'syndrome' part. He never told us it was a thing. That led me to (the Internet) and click, click, click, I found out about the hospital in Philadelphia."

After a tussle with the family's insurance company, Delaney was formally diagnosed by Sherry in January 2007 and put on a waiting list. It took three months before she began the program in Philadelphia.

"It was pretty terrible," Delaney says. "It was the hardest thing I've ever done in my life. It was mostly weight-bearing exercises. After treatment, I'd have these bruises from falling. If you didn't keep improving your time, you had to do it again. They didn't care how many times you had to repeat it."

The theory behind the intensive physical therapy, Sherry writes, is "exercise helps to quiet the small nerve fibers on a molecular level. Exercise has profound effects on the central nervous system—reversing some of the effects of chronic pain."

Delaney's two-year ordeal seems like a bad dream to her now. But, in a way, it may have guide her toward a career.

"I think I might want to be a physical therapist," she says.

Source

October 26, 2008